Extramammary Paget’s disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% of vulvar cancers. The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding many aspects of this condition including the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. This extensive review takes a closer look at what is known about EMPD and the conclusions that have been drawn from this information. This article also provides a practical approach to patients with EMPD.
Extramammary Paget’s disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% to 2% of vulvar cancers. Its pathogenesis has been debated, but most cases are thought to arise as a primary intraepidermal neoplasm of glandular origin. A minority seems to represent intraepithelial spread of an underlying dermal adnexal malignancy or a regional neoplasm with contiguous epithelium.
The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding the prevalence of concurrent underlying adenocarcinoma or invasive EMPD, associated malignancies, and optimal treatment. Drawing conclusions has been difficult due to the limited number of case series, usually involving a small number of patients, which have used not only widely differing investigative approaches but also showed significantly variable results. This inconsistency can be explained in part by retrospective reviews combining multiple institutions and multiple surgeons, and the lack of uniformity in the immunohistochemical analysis of specimens. One of the largest multi-institutional series of vulvar EMPD cases to date provided retrospective data on 100 patients with this condition, but immunohistochemical analysis was not performed.
This article aims to summarize what is known about EMPD to date in the literature. In addition, based on current knowledge, an approach to diagnosis and treatment is elaborated to aid clinicians faced with patients presenting this disease.
Epidemiology
EMPD is a rare condition with only a few hundred cases reported in the world medical literature. Precise incidence is unknown. EMPD represents 6.5% of all cutaneous Paget’s disease and affects predominantly patients between 50 to 80 years of age, with a peak age of 65. Women and Caucasians are more commonly affected, although a male predominance seems to exist in Asia. The vulva remains the most frequently involved site with 65% of EMPD located in this area. Familial occurrence is rare, with six reports in the Japanese literature and one in the British literature.
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